Congenital absence of all or part of one or more fingers or toes is defined as Ectrodactyly. It is the word derived from Greek ektroma meaning “abortion” and dactyly meaning digits. Also called split hand or foot malformation (SHFM), Ectrodactyly is a rare limb malformation with median cleft of hand and foot and aplasia or hypoplasia of the phalanges, metacarpals and metatarsals. Irregular pattern of inheritance exists for ectrodactyly; may be autosomal dominant, autosomal recessive or X linked. In this condition, the median cleft of the hand gives the hand, the appearance of lobster claws- so also called ‘Lobster Claw Hand”. Ectrodactyly may be very commonly associated with other anomalies of face, eyes and skeletal system. We report a rare case of unilateral ectrodactyly of right hand .The present case is not associated with any other anomaly as described in various textbooks and reference articles. The details of etiology, diagnosis, genetic causes, association with other anomalies and treatment will be discussed.KEYWORDS: Ectrodactyly, Split hand or foot malformations, Median Cleft, Autosomal dominant, Autosomal recessive.